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CCN proteins as potential actionable targets in scleroderma
Author(s) -
Henrot Pauline,
Truchetet MarieElise,
Fisher Gary,
Taïeb Alain,
Cario Muriel
Publication year - 2019
Publication title -
experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.108
H-Index - 96
eISSN - 1600-0625
pISSN - 0906-6705
DOI - 10.1111/exd.13806
Subject(s) - ctgf , matricellular protein , fibrosis , cyr61 , inflammation , pathogenesis , scleroderma (fungus) , medicine , biology , immunology , cancer research , pathology , microbiology and biotechnology , extracellular matrix , receptor , growth factor , genetics , inoculation
Systemic sclerosis ( SS c) is a complex autoimmune connective tissue disease combining inflammatory, vasculopathic and fibrotic manifestations. Skin features, which give their name to the disease and are considered as diagnostic as well as prognostic markers, have not been thoroughly investigated in terms of therapeutic targets. CCN proteins ( CYR 61/ CCN 1, CTGF / CCN 2, NOV / CCN 3 and WISP 1‐2‐3 as CCN 4‐5‐6) are a family of secreted matricellular proteins implicated in major cellular processes such as cell growth, migration, differentiation. They have already been implicated in key pathophysiological processes of SS c, namely fibrosis, vasculopathy and inflammation. In this review, we discuss the possible implication of CCN proteins in SS c pathogenesis, with a special focus on skin features, and identify the potential actionable CCN targets.